Hyperpigmentation and Hypopigmentation of the Skin after Long Term PUVA Therapy

An electron microscopic study was performed to demonstrate the pathological changes induced by long term PUVA treatment in recalcitrant psoriasis. Three patients developed mottling (hyperpigmentation and hypopigmentation) during two to three years of treatment. Three different types of morphological changes were found: disarrangement of keratinocytes, clustering and stimulation of melanocytes and homogenization of papillary dermis. Furthermore, the superficial blood vessels were loaded with the same type of amorphous granular substance. These changes might be specific to PUVA treatment or they might occur only in patients with previous treatment with, e.g., arsenic, methotrexate, anthralin + UVB or a combination of these.     

Nail changes associated with chemotherapy in children

BACKGROUND: Large series of chemotherapy-induced nail changes in children have rarely been reported in the literature. OBJECTIVE: To study the pattern and onset of nail changes in cancer children receiving various chemotherapy regimens. METHODS: A cross-sectional study was performed on 30 paediatric patients (aged 1-17, mean 8.3 years), including 11 with acute lymphoblastic leukaemia, five with acute myeloid leukaemia, and others. RESULTS: Nail changes developed in 10 children during chemotherapy, five of whom had Muehrcke's lines, three Beau's lines, one Mees' lines and another had trachyonychia. There appeared to be no correlation between the pattern of nail alteration and the underlying cancer types or stages, or the regimens of chemotherapy. CONCLUSION: One third of the children with cancers developed nail changes associated with chemotherapy. Among them, Muehrcke's lines were the most common manifestation, which were unrelated to hypoalbuminaemia in our series.     

8-Methoxypsoralen-Induced Nail Pigmentation

Ten psoriatic patients taking 8-methoxypsoralen and sunlight exposure (PUVASOL). were observed for changes in the nails. Three patients developed nail pigmentation.     

Localized scleroderma-like lesions on the legs in bone marrow transplant recipients: association with polyneuropathy in the same distribution

Summary We report two patients who developed localized scleroderma-like lesions on the legs following bone marrow transplantation. These changes were associated with a polyneuropathy in the same distribution.     

Psoriatic alopecia/alopecia areata-like reactions secondary to anti-tumor necrosis factor-α therapy: a novel cause of noncicatricial alopecia

With the increasing use of anti-tumor necrosis factor α (anti-TNF) biologic drugs to treat autoimmune diseases, an expanding array of adverse reactions is emerging. Anti-TNF drug-induced alopecia is a less well-known side effect of this class of drugs. The aim of this study was to define the clinical and histopathological features of alopecia arising in the setting of anti-TNF therapy. Clinical and histopathological features of 3 patients who developed scalp alopecia during anti-TNF treatment were examined. Two of the 3 patients also developed psoriasiform lesions outside the scalp, and biopsies from both scalp and nonscalp sites were reviewed. Clinically, each patient had large scaly patches associated with the scalp alopecia. All scalp biopsies revealed psoriasiform epidermal features and alopecia areata-like dermal changes. Epidermal changes included acanthosis and confluent parakeratosis with neutrophils and frank pustules. Dermal changes included markedly increased catagen/telogen and miniaturized hairs and peribulbar lymphocytic inflammation. Numerous plasma cells and eosinophils were present in all cases. Biopsies from the nonscalp lesions showed psoriasiform changes and prominent eosinophils and plasma cells. Two patients showed significant improvement of the alopecia with topical treatment only. In conclusion, anti-TNF therapy-related alopecia may closely mimic psoriatic alopecia and alopecia areata but can be histologically distinguished from alopecia areata by epidermal psoriasiform changes and dermal plasma cells and from primary psoriasis by the presence of plasma cells and eosinophils. A correct diagnosis can enable effective treatment and, in some cases, allow anti-TNF therapy to continue.     

Photosensitivity in HIV-infected individuals

OBJECTIVE: To characterize photosensitivity in HIV-infected individuals using minimal erythema dosage (MED) UVA (ultraviolet A light) and UVB (ultraviolet B light) photoprovocation light testing. DESIGN: Prospective, controlled analytical study. SETTING: University of California, San Francisco, between March 1995 and January 1997. PATIENTS: 13 HIV-seropositive patients with clinical and pathological features consistent with photodermatitis, 13 HIV-seropositive patients with biopsy-proven eosinophilic foliculitis (EF), and 10 HIV-seropositive patients with CD4 (T helper cell) count below 200 cells/uL and no history of photosensitivity or EF. INTERVENTION: Each patient underwent MED testing for UVB. All 13 patients with suspected photodermatitis underwent full photochallenge testing with UVA and UVB for up to 10 consecutive week days. RESULTS: Mean MED to UVB in patients with clinical photosensitivity and EF was lower (p = 0.004 and p = 0.022 respectively) than that of patients without a clinical history of photodermatitis. There were no significant differences in mean CD4 count or Fitzpatrick skin type. Positive photochallenge tests (papular changes at site of provocative light testing) to UVB (9 of 13 patients) were much more common than reactions to UVA (3 of 13 patients) in the photodermatitis group. All patients with clinically active photodermatitis developed papular changes at the site of UVB photochallenge testing, but only 1 of 5 patients with photodermatitis in remission developed papular changes with UVB photochallenge testing. Seven of the 13 patients with photodermatitis had Native American ancestry. Photosensitive patients were commonly taking trimethoprim-sulfamethoxazole (TMP-SMX), but no more commonly than EF or control patients. CONCLUSIONS: Photosensitivity in HIV-infected individuals appears to be a manifestation of advanced disease. Most patients are sensitive to UVB. The most severely affected individuals are both UVB and UVA sensitive, and may show reactions to visible light. A significant Native American ancestry may be a risk factor for development of photodermatitis in patients with advanced HIV disease. Finally, patients with eosinophilic folliculitis may be subclinically photosensitive.     

Rapid progression of lentigo maligna to deeply invasive lentigo maligna melanoma. Report of two cases

Two patients had lesions of lentigo maligna that evolved into deeply invasive (level 4-5) lentigo maligna melanoma during a relatively short period (two years and four years, respectively). In both patients, the clinical impression of lentigo maligna had been difficult to confirm by histopathologic analysis until the invasive tumor had developed. Both patients were actively followed up during this period of evolution, with our intention of detecting any early changes suggestive of invasive melanoma. Since deep invasion developed despite close clinical supervision, a more aggressive approach to the treatment of lentigo maligna may be warranted.     

Barmah Forest viral exanthems

A series of five patients presented with eruptions beginning on the face. In each case the facial changes were characterized by urticated erythema with minimal epidermal change. The patients also demonstrated more widespread vesiculopapular, macular or purpuric eruptions. At presentation most patients were asymptomatic; however, several subsequently developed constitutional symptoms. Each of these patients was reactive for Barmah Forest virus immunoglobulin (Ig)M, and on repeat testing four were reactive for Barmah Forest virus IgG.     

Erosive pustular dermatosis of the scalp

We report six patients with a previously undescribed but characteristic pustular dermatosis confined to the scalp. All the patients were elderly women who developed chronic, extensive, pustular, crusted and occasionally eroded lesions of the scalp which produced scarring alopecia. Investigations were essentially negative and skin biopsies showed only non-specific changes of atrophy and chronic inflammation, sometimes with increased plasma cells in the infiltrate. The condition did not respond to antibiotics, but was suppressed by potent topical steroids.     

Muco-cutaneous changes during long-term therapy with hydroxyurea in chronic myeloid leukaemia

Hydroxyurea is an antimetabolite agent used in the treatment of myeloproliferative disorders and sickle cell anaemia. Although hydroxyurea is relatively well tolerated, adverse effects often involve skin and mucous membrane during long-term therapy. A group of 510 patients affected by chronic myeloid leukaemia from 1977 to 1998 has been considered. Only 158 patients were treated with hydroxyurea and fulfilled inclusion/exclusion criteria of this study. A spectrum of severe cutaneous and mucosal changes (inflammatory and neoplastic) was seen in about 13% of patients (21 patients out of 158) and was studied in detail. Cutaneous and mucosal atrophy were observed in all 21 patients. Skin atrophy was often characterized by numerous telangiectases, especially on legs and on sun-exposed sites (16/21). Cutaneous, mucosal and nail hyperpigmentation was evident, albeit with variable extent, in 10 of the 21 patients. Severe stomatitis and glossitis with flattening of papillae were another common finding. Five patients, who received a particularly long treatment with hydroxyurea, developed squamous-cell neoplasms on sun-exposed sites (both squamous-cell carcinomas and keratoacanthomas). Acral changes were characteristic and constant, including acral erythema (21/21), dermatomyositis-like changes on the dorsa of hands (7/21), ulcers localized on acral areas of legs, on genitalia and oral mucosae (20/21). The frequency and the variety of these muco-cutaneous changes are reported and the mechanisms by which hydroxyurea may induce this muco-cutaneous syndrome-like group of changes, are proposed.     

Thalidomide-induced peripheral neuropathy. Effect of serum factor on nerve cultures

Sensory neuropathies developed in three of four patients with prurigo nodularis who had been treated with thalidomide. The serum samples of the patients who had neuropathy produced morphologic changes in cultured dorsal root ganglion cells. These observed changes support the postulate that thalidomide induces primary neuronal degeneration.     

Clinical Efficacy and Side Effects of Acitretin on the Disorders of Keratinization: A One-year Study

Acitretin, Ro 10-1670, the principal and free acid metabolite of etretinate, was used to treat twenty patients with disorders of keratinization. An open, prospective study of clinical efficacy, tolerability, and the effects of acitretin on lipid metabolism, hepatic function, and the osteoarticular system was performed over a one year period. Each patient was initially treated with 30 mg/day of acitretin or approximately 0.6 mg/kg/day. Doses were adjusted according to the clinical efficacy and maintained for one year. There were no statistically significant changes in liver function tests or lipid profile. Twelve of eighteen evaluated patients developed asymptomatic skeletal changes; the most common change was disc space narrowing, especially at thoracic-spine level (7 of 18 patients). The earliest bone change was detected 9 months after treatment. Acitretin is effective in improving the disorders of keratinization with mild mucocutaneous side effects and asymptomatic osteoarticular changes.     

Eosinophilic fasciitis associated with myositis: Report of four cases and review of the literature

Eosinophilic fasciitis is a rare scleroderma-like syndrome with an unknown aetiology. The characteristics of this disorder include lymphoplasmacytic inflammation involving the subcutaneous fat septa and fascia. Eosinophilic myositis is diagnosed when inflammation extends into the muscles. Here, we describe four patients who developed eosinophilic fasciitis, three of whom developed eosinophilic fasciitis with myositis. Fascial and muscle biopsies were used to confirm the diagnoses. All the patients presented with musculoskeletal symptoms; their electromyographic examinations showed myogenic lesions [short-duration, low-amplitude and polyphasic motor unit action potentials (MUPs), so-called myopathic changes, frequently with abnormal spontaneous activity], in contrast with findings from other reports.     

Pseudoxanthoma-elasticum-like skin changes induced by penicillamine.

The interference of penicillamine with collagen and elastin cross-linking can lead to wrinkling and anetoderma-like lesions in flexural areas as well as fragility and hemorrhagic blisters in pressure areas. These changes are seen primarily in patients with Wilson's disease or cystinuria who are on long-term therapy. This is a report of a patient with cystinuria on long-term, high-dose penicillamine who developed pseudoxanthoma-elasticum-like lesions. Coalescent yellow papules with a 'plucked-chicken skin' appearance were seen in the axillae and on the neck while redundant skin folds were noted in the anterior axillary line and lower buttocks. By light and electron microscopy, involved and uninvolved skin demonstrated 'lumpy-bumpy' dermal elastic fibers with no calcium deposition. These histologic changes are similar to those previously described in patients with penicillamine-induced skin lesions.     

Livedo racemosa generalisata: an evaluation of thirty-four cases

The results of investigations in 34 patients (28 women, 6 men) with livedo racemosa generalisata are presented. Neurologic or psychiatric symptoms were present in 28 patients. Nineteen patients had had one or more cerebral infarctions, and epilepsy (Sneddon's syndrome) developed in six. In most cases livedo preceded the neurologic disorder. In addition, many patients with livedo racemosa generalisata had Raynaud's phenomenon, cardiac abnormalities, or vascular changes in the ocular fundus.     

High melanosome engulfing activity of cutaneous fibroblasts in macular amyloidosis: an electron microscopic study

Six patients with macular amyloidosis were investigated by electron microscopy and various morphological changes in fibroblasts were identified. Many cells, which seemed to be macrophages by light microscopy, proved to be fibroblasts. The digesting action of fibroblasts was well developed and many melanosomes were taken into the cells making the cells resemble melanophages. The fibroblasts extended long, thin and branched cytoplasmic processes to surround the amyloid mass, and the cells selectively extended these into the narrow spaces between collagen and amyloid. The fibroblast showed highly developed endocytotic activity and probable ingcstion of amyloid by pinocytosis or phagocytosis. The developed rough cndoplasmic reticulum contained much protein and was active in secretion.     

Small pustules in Kawasaki disease. A clinicopathological study of four patients

We report the clinical and histological changes of small pustules that developed in four patients with Kawasaki disease (KD). The small pustules were superimposed on the urticarial erythema and symmetrically arranged on the genital area, buttocks, axillae, and extensor surface of the extremities. These lesions showed spongiform pustules histologically and were different from miliarial pustules. Reviewing previous reports, the clinical and histological characteristics of pustules in KD are summarized herein. This study indicates that small pustules underwent the consecutive changes related to the generalized polymorphous exanthem in KD.     

银屑病患者抗肿瘤坏死因子α治疗前后血清抗核抗体、抗dsDNA抗体及抗ENA抗体的变化

目的：观察银屑病患者接受抗肿瘤坏死因子α制剂治疗后抗核抗体（ANA）、抗dsDNA抗体和抗可提取性核抗原（ENA）抗体的变化。方法回顾分析32例银屑病患者,其中13例使用英夫利西单抗治疗,19例使用依那西普治疗。英夫利西单抗组第0、2、6周各用药1次,此后每隔8周用药,于每次用药前检测患者ANA、抗dsDNA抗体及ENA的情况和临床症状的变化。依那西普组每周用药2次,每3~6个月检测患者ANA、抗dsDNA抗体及ENA的情况和临床症状的变化。采用银屑病皮损面积和严重度指数（PASI）75、疾病活动评分（DAS）28评估临床疗效,间接免疫荧光法检测血清ANA水平,免疫印迹法和ELISA法检测抗dsDNA抗体水平,免疫印迹法检测抗ENA抗体水平。结果32例银屑病患者临床症状有不同程度缓解。32例抗TNF?α治疗的患者中有7例（21.9%）出现自身抗体,其中英夫利西单抗组中4例治疗（8.3±5.1）个月后出现自身抗体,3例ANA阳性,3例ENA阳性;依那西普组中3例治疗（9.0±3.0）个月后出现自身抗体,3例ANA阳性,1例ENA阳性。结论部分银屑病患者接受抗肿瘤坏死因子α制剂治疗后可出现自身抗体。     

Hydroxyurea and macrocytosis

Nine patients who were treated for psoriasis with hydroxyurea displayed peripheral macrocytosis. Mild megaloblastic changes were also seen in the bone marrow of three patients. One patient receiving hydroxyurea developed mild anaemia, another leukopenia. Macrocytosis did not appear to be secondary to an alteration in serum B₁₂ or folate levels. The haematological side-effects of hydroxyurea therapy, at the relatively low doses used to treat psoriasis, are similar to those seen with the higher doses used for the treatment of malignant tumours or leukaemia.     

Chlorella Photosensitization

ABSTRACT: Swelling followed by erythematopurpuric lesions on sun-exposed areas of the body developed in five patients. All patients were found to have ingested chlorella. The histopathologic changes consisted of swelling of endothelial cells and thrombosis of small blood vessels in the dermis and the subcutaneous fatty tissue. The photosensitizing agent contained in chlorella tablets was proved to be pheophorbide-a and its ester.